肿瘤相关噬血细胞综合征的诊治

王昭; 王晶石

doi:10.13201/j.issn.1004-2806.2023.01.001

肿瘤相关噬血细胞综合征的诊治

王昭^1, ,,
王晶石¹

- 首都医科大学附属北京友谊医院血液科(北京，100050)
基金项目:
国家自然科学基金(No：81871633)；首都卫生发展科研专项重点攻关项目(No：首发2020-1-2022)

详细信息

作者简介:
王昭，医学博士、教授、主任医师、博士研究生导师。现任首都医科大学附属北京友谊医院血液内科主任，国际组织细胞协会噬血细胞综合征分会执行委员，中国医师协会血液科医师分会组织细胞疾病专业委员会主任委员，中国抗癌协会血液肿瘤专委会EB病毒工作组组长，中华医学会血液学分会淋巴细胞疾病学组副组长。长期致力于噬血细胞综合征相关临床基础研究，自主研发早期诊断关键技术，获批国家发明专利授权9项，为精准治疗提供依据。研究成果以第一完成人获得中华医学科技奖二等奖、华夏医学科技奖二等奖、北京医学科技奖二等奖、中国“金桥奖”二等奖、北京市科学技术奖三等奖。以第一与通信作者在国内外学术期刊上发表论文260余篇

通讯作者: 王昭，E-mail：wangzhao@ccmu.edu.cn

中图分类号: R55

收稿日期: 2022-11-19

刊出日期: 2023-01-01

Diagnosis and treatment of malignancy-associated hemophagocytic lymphohistiocytosis

WANG Zhao^1, ,,
WANG Jingshi¹

- Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing, 100050, China

More Information

Corresponding author: WANG Zhao, E-mail: wangzhao@ccmu.edu.cn

Received Date: 19 November 2022

Publish Date: 01 January 2023

摘要

摘要: 噬血细胞综合征是一种由各种诱因导致的细胞毒性T细胞、自然杀伤细胞和巨噬细胞异常激活，增殖失控，释放大量炎性因子引起的过度炎症反应综合征。肿瘤是导致继发性噬血细胞综合征的重要病因之一，发病率随着年龄的增长而增高。肿瘤相关噬血细胞综合征起病凶险，临床症状错综复杂，易致误诊、漏诊，死亡率高。近年来，随着对疾病认识的不断提高，肿瘤相关噬血细胞综合征的诊断和治疗策略不断更新，但仍缺乏大规模前瞻性临床研究指导疾病的规范化诊断和治疗。随着越来越多新药的涌现和临床研究的陆续开展，肿瘤相关噬血细胞综合征的诊断和治疗将不断完善，最终达到改善患者预后的目的。
- 肿瘤相关噬血细胞综合征 /
- 诊断 /
- 治疗
Abstract: Hemophagocytic lymphohistiocytosis(HLH) is a critical condition characterized by the undue activation of cytotoxic T lymphocytes, natural killer cells, and macrophages, resulting in excessive secretion of infammatory cytokines. Malignancy is an important trigger of secondary HLH, and the incidence of malignancy-associated HLH(M-HLH) increases with age. M-HLH is a life-threatening disease with an interdisciplinary complex of symptoms, high incidence of misdiagnosis and mortality. In recent years, with increasing awareness to this disease, the diagnosis and treatment of M-HLH have been renewed constantly. However, there is still no consensus on the diagnostic criteria and treatment strategies due to lack of large-scale prospective studies. With emerging drugs and ongoing clinical trials, it's worth believing that the diagnosis and management of M-HLH will be optimized, which will ultimately improve prognosis.
- malignancy-associated hemophagocytic lymphohistiocytosis /
- diagnosis /
- treatment

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